Congenital diaphragmatic eventration and hernia sac compared to CDH with true defects: a retrospective cohort study.

Congenital diaphragmatic eventration (CDE) and congenital diaphragmatic hernia (CDH) with or without hernia sac are three different types of congenital diaphragmatic malformations, which this study evaluates. All surgically treated patients with CDE or Bochdalek type CDH between 2000 and 2016 were included in this retrospective analysis. Demographics, CDH-characteristics, treatment, and clinical outcome were evaluated. In total, 200 patients were included. Patients with an eventration or hernia sac had no significant differences and were compared as patients without a true defect to patients with a true defect. The 1-year survival of patients with a true defect was significantly lower than patients with no true defect (76% versus 97%, p = 0.001). CDH with no true defect had significantly better short-term outcomes than CDH with true defect requiring patch repair. However, at 30 days, they more often required oxygen supplementation (46% versus 26%, p = 0.03) and had a higher recurrence rate (8% versus 0%, p = 0.006) (three eventration and two hernia sac patients). Conclusion: Patients without a true defect seem to have a more similar clinical outcome than CDH patients with a true defect, with a better survival. However, the recurrence rate and duration of oxygen supplementation at 30 days are higher than CDH patients with a true defect.What is Known:• Congenital diaphragmatic hernia with or without hernia sac and congenital diaphragmatic eventration (incomplete muscularization) are often treated similarly.• Patients with hernia sac and eventration are thought to have a relatively good outcome, but exact numbers are not described.What is New:• Congenital diaphragmatic eventration and patients with hernia sac seem to have a more similar clinical outcome than Bochdalek type CDH with a true defect.• Patients without a true defect (eventration or hernia sac) have a high recurrence rate.

Congenital eventration of the diaphragm in children: 25 years' experience in three pediatric surgery centers.

Eventration of the diaphragm is generally defined as an abnormal elevation of all or a portion of an attenuated but otherwise intact diaphragmatic leaf. Previous studies have indicated that eventration is a relatively rare condition, which can be symptomatic and requires surgery. We aimed to evaluate our patients with diaphragmatic eventration, and to discuss their characteristics in the light of the relevant literature. We retrospectively analyzed age, sex, incidence, location of the eventration, symptoms, associated anomalies, surgical technique, complications and survival in our patients. Between 1974 and 1999, 33 patients were treated in the Pediatric Surgery Departments of Ege University, SSK Children's Hospital and Behçet Uz Children's Hospital, 18 of them boys and 15 girls. The ages of our patients ranged from three days to 12 years. All of the patients had at least one of the respiratory symptoms such as cough, respiratory distress and fever; 3 newborns were admitted with severe respiratory distress while children belonging to higher age groups had symptoms of acute or recurrent pulmonary infections and failure to thrive. The eventration was right-sided in 22, 11 eventrations were left-sided. Diagnosis was performed with the help of a number of radiological studies such as fluoroscopic investigation, contrast study of the upper gastrointestinal system, direct X-ray graphies of the thorax, CT scan and ultrasonography, as necessary. Surgery was performed via thoracotomy in 20 patients and the 12 other patients underwent laparotomy for plication. One patient underwent thoracoabdominal plication. Two patients died because of cardio-respiratory complications in the early postoperative period and the rest of them survived to annual follow-ups. In conclusion, diaphragmatic eventration is an important condition which can eventually be mortal. Early diagnosis is necessary and plication is the treatment of choice.

Long-term gastrointestinal morbidity in patients with congenital diaphragmatic defects.

Recent reports indicate a significant incidence of gastroesophageal reflux (GER) and other nonpulmonary problems after the repair of congenital diaphragmatic defects. Reports of follow-up through adulthood are few and based on a small number of patients. From 1948 to 1982, 107 of 164 patients (65%) treated at the authors' institution survived after repair of congenital diaphragmatic hernia or eventration. Sixty of the 107 survivors (56%) (mean age, 29.6 years; SD, 9.0 years) were interviewed and examined clinically. Forty-one of the sixty (68%) underwent upper gastrointestinal endoscopy. Early postoperative GER was recorded for 11 of the 60 patients (18%). Two of them underwent fundoplication because of an esophageal stricture. At the time of the follow-up study, 38 of the 60 (63%) reported symptoms suggestive of GER. Endoscopic or histological GER (esophagitis, Barrett's esophagus) was present in 22 of 41 patients (54%). No significant correlation between the initial severity of the diaphragmatic defect or neonatal postoperative problems and the late GER could be verified. Intestinal obstruction requiring hospital admission occurred in 12 of the 60 patients (20%), eight of whom had surgical treatment between 1 month and 20 years after repair of the diaphragmatic defect. GER and intestinal obstruction are common among patients who have undergone repair of a congenital diaphragmatic defect. Investigations for GER should be performed routinely during the follow-up of these patients.

Patterns of malformation in children with congenital diaphragmatic defects.

The medical records of 102 live-born children with a congenital diaphragmatic defect were reviewed to determine the frequency and nature of underlying chromosomal, genetic, and nongenetic patterns of malformation. Overall, 40 children (39%) had a major nonpulmonary malformation, and 14 of these children (14%) had a previously recognized pattern of malformation. A group of 18 children (18%) with cardiac anomalies had an increased mortality rate in comparison with those children without cardiac defects (72% vs 38%). The frequency and severity of nonpulmonary abnormalities in children with congenital diaphragmatic defects suggest that examination of affected children should include cardiac evaluation, a karyotype when the defect is one feature of a broader pattern of altered development, and a careful evaluation for minor anomalies, which may provide clues to an overall diagnosis.

[Congenital diaphragmatic hernia. 120 neonatal cases. Preliminary study]. / Hernies diaphragmatiques congénitales: 120 cas néo-nataux. Etude préliminaire.

120 newborns with a congenital diaphragmatic hernia (CDH) were treated during the first day after birth in the Department of Pediatric Surgery at Saint Vincent de Paul's Hospital from 1980 to 1987. The overall survival rate was 40%. The influence of prenatal diagnosis, clinical and anatomical aspects is analysed. The side and the size of the defect, the intra-thoracic liver, the intestinal malrotation and the abdominal hypoplasia are significant prognosis factors. This preliminary study suggests that the natural history of CDH may be responsible of two different diseases: a disease with an early visceral intra-thoracic growth, and a severe lung hypoplasia; another one with a secondary herniation of the intra-abdominal mass, and a better prognosis.